Mental health difficulties in children with learning ... · •Di George •Down’s •Fragile X...

Mental health difficulties in children with

learning disabilities

Chris Oliver and Jane Waite

University of Birmingham

Aston University

The problems• Substantial impairment of adaptive behaviour (day to day support for ADL) and

limited speech\expressive communication

• What is included under ‘mental health’

– Challenging behaviour

– ‘Comorbid’ ASD and ADHD

– Impulsivity and repetitive behaviour

• The ‘value’ of psychiatric taxonomy

– Grossly atypical development of CNS

– Additional impairment

• Aetiology and psychiatric taxonomy

– VCFS, TSC, PWS (UPD) but… ASD in FXS, RTS and CdLS, anxiety in WS

• Diagnostic criteria

– Speech (language) and thought

– Identify, label, report

– Measure

• In combination these factors militate against identification of a mental health problem

Outline

• Cause matters

• Physical health (pain, discomfort, sleep)

• Learned behaviour

• ADHD and ASD

• Anxiety and low mood

The normal distribution of IQ scores and the basis to the two group approach

Some genetic syndromes associated

with intellectual disability

•Aarskog

•Addison-Schilder

•Aicardi syndrome

•Alagille syndrome

•Allan-Herndon-Dudley

•Alpha thalassemia

•Alport

•Angelman

•Aspartylglycosaminuria

•Bardet-Biedl

•Beckwith-Weidemann

•Bertini

•Bickers-Adams

•Bloch-Sulzberger

•Brunner

•Cardiofacial

•Carpenter

•Cat eye

•CHARGE

•Christian syndrome

•Cleidocranial dysplasia

•Cohen

•Cornelia de Lange

•Cowchock

•Cri du chat

•Di George

•Down’s

•Fragile X

•Fucosidosis

•Garcia-Lurie

•Goltz-Gorlin

•Greig-cephalopolysyndactyly

•Heterotaxia

•Hischsprung disease

•Hunter

•Hurler

•Kabuki make-up

•Kallmann

•Lesch-Nyhan

•Lowe

•Mandibulofacial dysostosis

•Marsidi

•Pateau

•Perlman

•Pitt-Rogers-Danks

•Prader-Willi

•Rett

•Richner-Hanhart

•Rieger

•Rubinstein-Taybi

•Rud

•Shprintzen

•Shprintzen-Goldberg

•Silver-Russell

•Smith-Magenis

•Snyder-Robinson

•Sotos

•Usher

•Watson

•Williams

•Wolcott-Rallison

•Wrinkly skin

•Zinsser-Engman-Cole

Significant loss or change of genetic information caused by:

•Numerical chromosome abnormality (e.g. Down syndrome)

•Structural chromosome abnormality (e.g. Cornelia de Lange, Angelman, Prader-Willi

syndromes)

•Single gene disorders (e.g. Fragile X syndrome)

Self-

injury

Temper

outbursts

Motivation

for social

contact

Sleep

disorder

Impulsivity

Smith-

Magenis+++ +++ +++ +++ +++

Angelman - - ++ +++ ++

Prader-Willi + +++ + ++ -

Cornelia de

Lange++ - - + +

Cri du Chat + - ++ ++ ++

Complexity and effects on the environment

Outline

• Cause matters

• Physical health (pain, discomfort, sleep)

• Learned behaviour

• ADHD and ASD

• Anxiety and low mood

0

5

10

15

20

25

No self-injury Self-injury

GR

Q S

co

re

Reflux related behaviours in CdLS

(p<.01)

Cornelia de Lange syndrome: Self-injurious behaviour, gastro-

intestinal disorders, middle ear infections, dental abnormalities and

disorders

Pain and discomfort

– More pain behaviours in those showing self-injury?

– Higher prevalence of self-injury in those with health problems?

– More self-injury in those with suspected health problems

0

5

10

15

20

25

30

Self-injury No self-

injury

Me

dia

n N

CC

PC

-R s

co

re

Comparison of pain behaviours in children

with Tuberous Sclerosis Complex

(U=27; p<.001)

Kate Eden, Cerebra PhD studentship holder

Cause may vary

Pain and discomfort

– More pain behaviours

in those showing self-

injury?

– Higher prevalence of

self-injury in those

with health problems?

– More self-injury in

those with suspected

health problems

0

1

2

3

4

5

6

Children Adults

Re

lati

ve

ris

k

Relative risk of frequent self-injury in

children and adults with ASD given the

presence of health problems

(99% CI)

Caroline Richards, PhD

Pain and discomfort• The assessment of pain

– FLACC (Merkel et al., 1997)

– NCCPC (Breau et al., 2004)

– QABF (Paclawskyi et al., 2000)

• Behavioural correlates of pain and challenging behaviour:

– More pain behaviours in those showing challenging behaviour?

– Higher prevalence of challenging behaviour in those with health problems?

– More challenging behaviour in those with suspected health problems

• Social\operant causes and pain behaviour?

• New directions in assessment– Temporal relationships

Kate Eden, Cerebra PhD studentship holder

www.findresources.co.uk

Pain and discomfort• The assessment of pain

– FLACC (Merkel et al., 1997)

– NCCPC (Breau et al., 2004)

– QABF (Paclawskyi et al., 2000)

• Behavioural correlates of pain and challenging behaviour:

– More pain behaviours in those showing challenging behaviour?

– Higher prevalence of challenging behaviour in those with health problems?

– More challenging behaviour in those with suspected health problems

• Social\operant causes and pain behaviour?

• New directions in assessment– Temporal relationships

Kate Eden, Cerebra PhD studentship holder

Other outrageously

expensive smartphones

are also available

“(……..) only has challenging behaviours when in pain. This results in a

complete change in personality, ripping lumps of hair out so massive patches are

missing. screaming like a banshee.

But we are not believed at hospital and just get sent home as they see no fever,

no infections, ears, eyes, teeth, skin, joints. And refuse to do anything even basic

bloods or x-rays. We then have to go to our community consultant who found

that acid reflux had burned her severely and finally got meds needed. The

hospital telling us that she had nothing wrong and it was behavioural or

neurological. ………

Is this pain tool going to be any use to use if no one listens?”

Parent of a child with Angelman Syndrome

“There are at least 33 syndromes of learning disabilities where

a behavioural phenotype has been reported……. the

mechanism by which a genetic disorder could cause …….

behaviours is largely unknown, the ultimate pathway must be

the structure and the function of the brain. Most of these

behaviours are not curable………...”

Psychiatry text published in 1996

Behavioural Phenotypes and Genetic Determinism

• Prevalence estimates of 1 in 25,000 births (Greenberg et al., 1991) to 1 in 15,000 (Laje et al., 2010)

• Deletion chromosome 17 p11.2 (Greenberg et al., 1991; Smith et al., 1986)or mutation (gene RAI1) (Slager et al., 2003)

Smith Magenis syndrome

Effects of treating reflux on self-injury, Mood, Pain & Sleep

Pre-Treatment 1 Post-Treatment 1

Challenging Behaviour Interview 31/55 28/55

Frequency Daily Daily

Worst Effect Moderate injury(bruising, cuts, abrasions)

Moderate injury(bruising, cuts, abrasions)

Mood, Interest and Pleasure Questionnaire

30 36

Gastroesophageal Distress Questionnaire Total Score

45 39

FLACC (average across 5 days) 4 0.6

Total Sleep Time (average across 5 days)

06:49:24 07:15:00

Number of Night Wakings(average across 5 days)

1.8 (range = 1-4) 1.4 (range = 1-2)

Total Waking Duration 01:35:00 (range = 00:10 –06:05)

00:15:00 (range = 00:05 –00:30)

Outline

• Cause matters

• Physical health (pain, discomfort, sleep)

• Learned behaviour

• ADHD and ASD

• Anxiety and low mood

ENGAGE

Comfort

Reprimand

Offer

Restrain

Occupy

Distract

ACTION

SIBConcern

Frustration

Anxiety

Confusion

Distress

AVERSIVE!

Positive

Reinforcement

REWARD

Increase in

chance of CB

Social Communicative Function of Challenging

Behaviour: Positive Reinforcement

Need for others to

do or give

something

Outline

• Cause matters

• Physical health (pain, discomfort, sleep)

• Learned behaviour

• ADHD and ASD

• Anxiety and low mood

Autism Screening Questionnaire

% ASD

% Autism

Social Comm. Rep. Beh.

Angelman (15q11-q13)

66.3 17.8 ++ + -

Cri du Chat (5p 15.2-15.3)

40.0 8.0 ---- ----- -

Cornelia de Lange (5p 13.1)

78.8 45.9 ++ + O

Fragile X (Xq27.3)

83.6 46.3 ++ ++ ++++

Prader-Willi (15q11-q13)

45.8 15.5 ---- - --

Lowe (Xq26.1)

71.2 34.6 O + +

Smith Magenis (17p 11.2)

68.4 36.8

O O ++

Age range 4 to 54

+ indicates score higher than 1 other group, - indicates score lower than 1 other group, O indicates no

difference from any other group.Oliver, C. et al. (2011). JADD, 41, 1019-1032

Autism Spectrum Disorder (ADHD)

or not?

• Behaviourally defined, list of criteria

– Attaining cut-off scores but with different item level

profiles

– Scoring on an item for different reasons

– Communication problems

• Is the diagnosis helpful?

– Services

– Good advice from Autism (ADHD) materials

Outline

• Cause matters

• Physical health (pain, discomfort, sleep)

• Learned behaviour

• ADHD and ASD

• Anxiety and low mood

Difference of emotion

Thornton, F. and Matthews, P. (2008). Addressing the balance. 1st Asia Pacific Prader-Willi syndrome

conference. Wellington, New Zealand.

Emotional

difference

Outline

• Cause matters

• Physical health (pain, discomfort, sleep)

• Learned behaviour

• ADHD and ASD

• Anxiety and low mood

Prevalence of anxiety and depression in people with intellectual disabilities

24% of young people and

children with ID

experience mental health

difficulties

Anxiety and depression

and mixed affective

disorder are the most

common diagnoses.

3-22% of children with

intellectual disabilities

have an anxiety disorder (Reardon et al., 2015).

Four to six times more

likely to have an affective

disorder (Taylor et al., 2004).

What increases the prevalence?

Person characteristics

• Information processing (rate and

complexity).

• Reduced executive functioning:

memory, inhibition, flexibility,

problem-solving and planning,

predicting the future.

• Low levels of adaptive skills

Altered processing of

information i.e. attention to

more threatening stimuli.

Fewer cognitive resources

in the face of stressorsHout et al., 2009; Taylor & Knapp, 2003

Intolerance of uncertainty.....

Boulter et al. (2014); Wigham et al. (2015)

Interconnected set of

neurobiological and

psychological processes

Cognitive capacity to

manage uncertainty is

reduced

Typically developing

Intellectual

disabilityAutismRare genetic

syndromes

(e.g. Williams,

fragile-X or

Cornelia de

Lange syndromes

Royston et al., 2016

Why are anxiety and depression more

prevalent?

Environment factors around the person

• Boredom

• Loneliness

• Lack of opportunity to exert

control over own life and the

future

• Lack of meaningful friendships

and relationships

• Stressful family circumstances

• Stigmatisation and bullying

• Being asked to complete tasks

that are too difficult and

opportunities being removed

• Unemployment

• Debt

• Chronic poverty

Matorell et al., 2009; MacMahon & Jahoda, 2008

Anxiety and depression remain

undetected, and hence untreated

Therapeutic Disdain

Psychological

therapies are

ineffective with people

with ID

Lack of clinical

research in this area

Therapeutic overshadowing

Social withdrawal being

seen as a lack of social

skills rather than depression

or anxiety

Crying as an indicator of

pain rather than depression

Lack of engagement with

activities as being due to

intellectual disability

Ethos of services

focusing on challenging

behaviour above mental

health

Therapeutic overshadowing

Social withdrawal being

seen as a lack of social

skills rather than depression

or anxiety

Crying as an indicator of

pain rather than depression

Lack of engagement with

activities as being due to

intellectual disabilityInaccurate

identification works

both ways!

Social withdrawal being

diagnosed as depression

when it is Autism

Spectrum Disorder

Pain rather than depression

Different interests and

desires, rather than

depression

0

25

50

Perc

en

tag

e

Prevalence of Selective Mutism

Social anxiety in CdLS

– High levels of anxiety associated with social situations.

– Strong preference to observe rather than participate.

– Increased withdrawal when social demands become heightened.

– Motivation for social contact appears to be intact.

Moss et al., 2008. AJMR 113, 278-291;

Richards et al., 2009, JADD, 39, 1155.

Reid, Nelson , Moss & Oliver, In preparation

Inadequate assessment measures

Based on the general population. Different behaviours? (Fydrich et al.,

1998; Rodgers et al., 2012)

Even less appropriate for people with severe intellectual disability

Do not interrogate behaviour change

Do not rule distinguish physical and emotional distress

Anxiety

Signs

DepressionActivity Levels

Social engagement

Interest and pleasureSelf care

skills/adaptive skills

Eating

SleepingEmotional expression

Range of facial expressions

Change???

Working with families and

staff team to develop their

awareness of mental health

The immediate environment of the person

is a legitimate target for intervention

Making environment more

sensitive to the individual’s

needs

Increasing social engagement

and engagement (or reducing

this if it is not reinforcing to

the person)

Increasing engagement in

meaningful activity that

provide natural reinforcement

Increasing an individual’s

repertoire of functional skills

Jones & Dowey, 2013

Improving quality of life

Increase

controllability and

choice!

Carr et al. (2009)

Focus on communication

Give a choice

Increasing certainty!

•Predictable routines

•Visual timetables

•Using a cue card when change occurs

Increasing tolerance to

uncertainty (building

skills!)

-Scheduling something

unpredictable

-Introducing subtle

changes

-Skills to cope with

stress

-Relaxation Training

- Skills development (ways of coping a difficult situation)

- Graded exposure (Modelling/Rewards) for anxiety

- Behavioural activation for depression

Although Cognitive Behaviour Therapy may be appropriate for

some people with ID

Chalfant et al 2007; Dagnan et al., 2005

Psychological therapy is not just ‘talking

therapy’

Summary

Anxiety and depression are often overlooked in people with intellectual disability.

Individuals with intellectual disability experience more adverse life events and may be equip with fewer skills to manage these difficulties.

Assessment remains problematic due to difficulties with:

• Self-report and parental-report

• Confounded measurement tools (ASD, challenging behaviour, pain)

Assessing change from baseline is key.

A goal of psychological intervention is to increase access to activities and environments that encourage well-being, social contact and meaningful friendships.

Closing thoughts

• Cause matters (check syndrome information)

• Physical health (pain, discomfort, sleep)

• Learned behaviour

• ADHD and ASD (a pragmatic approach)

• Anxiety and low mood (underestimated?)

Core Funding

Cerebra

Grant SupportMedical Research Council

The Big Lottery

Baily Thomas Foundation

Cornelia de Lange Syndrome Foundation

Research Autism

Birmingham Children’s Hospital

Angelman Syndrome Foundation (USA)

Newlife

National Autistic Society

Economic and Social Research Council

Jerome Lejeune Fondation

Tuberous Sclerosis Association

NIHR

Leverhulme

j.waite@aston.ac.uk

c.oliver@bham.ac.uk

www.researchgate.net

www.findresources.co.uk